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1 Departments of Radiologic Pathology (J.K.M., P.T.R., G.N.B., N.K., P.R.R.)
2 Hepatobiliary Pathology (Z.D.G.), Armed Forces Institute of Pathology, 14th and Alaska Sts, NW, Washington DC 20306
3 Department of Radiology, Okayama University, Japan (N.K.)
4 Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, Mass (P.R.R.)
Fibrolamellar carcinoma is a malignant hepatocellular tumor with distinct clinical and pathologic differences from hepatocellular carcinoma. It differs from hepatocellular carcinoma in demographics, condition of the affected liver, tumor markers, and prognosis. Fibrolamellar carcinoma characteristically manifests as a large hepatic mass in adolescents or young adults (without gender predominance). Cirrhosis; elevated
-fetoprotein levels; and typical risk factors for hepatocellular carcinoma such as viral hepatitis, alcohol abuse, and metabolic disease are typically absent. Fibrolamellar carcinoma is characterized pathologically by cords of tumor cells surrounded by abundant collagenous fibrous tissue arranged in a parallel or lamellar distribution. Fibrotic lamellae often coalesce to form a central scar. Fibrolamellar carcinoma characteristically appears on radiologic images as a lobulated heterogeneous mass with a central scar in an otherwise normal liver. Radiologic evidence of cirrhosis, vascular invasion, or multifocal diseasefindings typical of hepatocellular carcinomais uncommon in fibrolamellar carcinoma. Imaging features of fibrolamellar carcinoma overlap with those of other scar-producing lesions including focal nodular hyperplasia (FNH), hepatocellular adenoma and carcinoma, hemangioma, metastases, and cholangiocarcinoma. FNH, in particular, may simulate fibrolamellar carcinoma, since both have similar demographic and clinical characteristics. Because some believe that radiologic diagnosis of FNH is possible, it is important to understand the imaging appearance of fibrolamellar carcinoma to avoid misdiagnosing this malignant tumor as a FNH.
Index Terms: Liver neoplasms, 761.324 Liver neoplasms, MR, 761.12141
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